J Dent Res Dent Clin Dent Prospects. 2009 Spring; 3(2): 70–72.
Amirala
Aghbali, 1 Firouz
Pouralibaba, 2
,*
Hossein
Eslami, 3 Farzaneh Pakdel, 3 andZahra Jamali 3
Abstract
Introduction
White sponge nevus (WSN) is a relatively rare cutaneous and mucosal
lesion.1,2,3 Hyde reported the first case of WSN in 1909 and a detailed report was
published in 1935 by Cannon.4,5 Etiologically, it is a rare developmental anomaly
inherited as an autosomal dominant trait with variable expressivity and a high
degree of penetrance. This condition is attributed to a defect in the normal
keratinization (keratin 4 and keratin 13, which are specifically expressed in the
spinous cell layer of the oral mucosa). 6 - 11 This keratotic mucosal alteration may
be seen on vaginal and rectal mucosa but the great majority of cases involve the
oral mucosa.1
A search of dermatological and gynecological literature revealed very little about
WSN in Iran. More information was available about this lesion in the oral cavity,
which was retrieved from the dermatological and dental literature.12
Lesions of WSN usually appear at birth or in early childhood, but sometimes the
condition develops during adolescence. The lesions consist of symmetric,
thickened, white, corrugated or velvety, diffuse plaques. Buccal mucosa is the most
frequently affected, followed by the labial and gingival mucosa, and the floor of the
mouth. Extra-oral mucosal sites, such as the nasal, esophageal, laryngeal, and
anogenital mucosa, appear to be less commonly affected. Patients are usually
asymptomatic. The white color does not diminish when the tissue is stretched in
any mucosal site. 1,2,6 ,13,14
The recognition of this disorder is important in that it must be differentiated from
other congenital or familial disorders of more widespread clinical significance. The
clinical appearance is so distinctive that biopsy is usually unnecessary. The
microscopic features of WSN are characteristic but not necessarily pathognomonic.
Prominent hyperparakeratosis and marked acanthosis with clearing of the
cytoplasm of the cells in the spinous layer are common features; however, similar
microscopic findings may be associated with leukoedema and hereditary benign
intraepithelial dyskeratosis. In some instances an eosinophilic condensation is
noted in the perinuclear region of the cells in the superficial layers of the
epithelium, a feature that is unique to WSN.1-5
In this paper, a case of WSN in a healthy white male with no history of familial
involvement is described.
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Case Report
The patient was a 21-year-old Iranian male referred to the Department of Oral
Medicine at Tabriz University of Medical Sciences Faculty of Dentistry for diagnosis
and management of a “white, itchy spot” on the buccal mucosa. White bilateral
lesion in oral mucosa was the chief compliant of the patient. The patient
complained of a white lesion which was present since birth.
The patient’s general health was reportedly good. The patient denied presence of a
similar condition in immediate family members or any similar lesions elsewhere on
his body.
In clinical examination, there were bilateral, symmetrical white plaques and
patches on the buccal and labial mucosa, which could not be removed (Figure 1).
The plaques were smooth with velvety texture and irregular, well-defined borders.
There was no elevation or erythema. The margins were clear and no lymph nodes
were noticeable. Oral hygiene was good and other oral structures were normal in
appearance.
Figure 1. Clinical views of the lesions.
In histopathologic evaluation, oral mucosa covered by stratified squamous
epithelium revealed prominent hyperparakeratosis and marked acanthosis with
clearing of the cytoplasm of cells in the spinous layer. In addition, eosinophilic
condensation was noted in the perinuclear region of the cells in superficial layers
(Figure 2). Underlying connective tissue was normal in appearance with rare
chronic inflammatory cell infiltration.
Figure 2. (a) Histopathologic view of the lesion (×10); (b) perinuclear
condensation of keratin tonofilament (arrow) (×40) (H&E).
a
b
Based on clinical data and histopathologic findings, the lesion was consistent with
white sponge nevus. Because of benign nature of this lesion, no treatment is
necessary and only biopsy and correct diagnosis is necessary to rule out other
similar lesions. Six-month follow-up was recommended.
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Discussion
WSN is a rare hereditary dyskeratotic hyperplasia of mucous membranes. This
entity is also known by other names, such as Cannon's disease, familial white
folded hypertrophy of the mucous membranes, hereditary leukokeratosis, white
gingivostomatitis, and exfoliative leukoedema.1-5WSN is an autosomal dominant
disorder with variable penetrance and hence familial reports are not very common,
similar to the present case. WSN has been listed as a rare disorder, with a
prevalence rate below 1 in 200,000.13 Most commonly, lesions appear at birth or in
early childhood. Neither gender nor racial predilection exists. 14 A case of WSN, in
which human papilloma virus type 16 was demonstrated, has been reported in the
literature.4 Many different types of white lesions can occur in the oral mucosa and
the appearance of WSN is not pathognomonic. There is a need for precise
identification through prompt histopathologic examination to differentiate this
condition from more serious, potentially premalignant lesions as well as other
genodermatoses such as hereditary benign epithelial dyskeratosis, lichen planus,
lichenoid drug reaction, lupus erythematosus, cheek chewing and possibly
candidiasis. While some of these lesions are benign, others are pre-malignant or
manifestations of some systemic diseases. Therefore, early diagnosis of this
benign lesion is important,3 and often, these lesions need different treatment
plans.15-21 In addition, these lesions reveal different epidemiological patterns and
involve different societies and races. In Northwest Iran, this condition seems to be
rare and no other similar documented cases are available. In this case, none of the
family members had similar lesions. This lesion appeared early in life without any
reported changes throughout the patient’s life, but diffuse spreading of the lesion
seems to be an alarming factor. Biopsy in such cases is necessary for treatment
planning and ruling out of other lesions.
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